肝型肝豆状核变性患者认知灵活性的研究

A study on cognitive flexibility in hepatic type hepatolenticular degeneration patients

目的通过神经心理学测验评估肝型肝豆状核变性（HLD）患者的认知灵活性变化特点；探讨肝型HLD患者认知灵活性功能改变的发生机制。方法选取36例肝型HLD患者为研究对象；正常对照组30例。正常对照组和HLD患者均详细记录性别、年龄、受教育年限以及瑞文标准推理测验（R＇SPM）、连线测验（TMT）的结果。结果HLD组在TMT的A项耗时[（35．94±11．51）s]、B项耗时数[（117．94±44．30）s]及干扰量[（82．42±43．49）s]上与对照组[分别为（32．31±7．25）s、（65．31±19．50）s、（33．00±15．17）s]比较差异有显著性（P〈0．05）；在R＇SPM的智商、类同比较、抽象推理测验分值上差异有显著性（P〈0．05）。HLD组TMT的B项耗时数、干扰量上与年龄、病程呈正相关（P〈0．05或P〈0．01）；R＇SPM的比较推理分上与年龄、病程呈负相关（P〈0．05）。结论肝型HLD患者认知灵活性存在功能缺陷，轻微型肝性脑病引发额叶-纹状体环路上功能受损可能是肝型HLD患者认知灵活性障碍的主要机制。

Objective To assess the characteristics of cognition flexibility in hepatic type hepatolenticular degeneration （HLD） patients through neuropsychological tests; and to research on the mechanism of cognition flexibility dysfunction in hepatic type HLD patients. Methods 36 hepatic type patients were researched as HLD group. 30 healthy people were selected as control group. All the patients and control group were recorded the sex, age, years of education and the test results of Raven＇ s standard progressive matrices （ R＇ SPM）, trail making test （TMT）. Results HLD group compared with control group were exceedingly significantly difference in A item time-consuming [ （35.94 ± 11.51 ） s], B item time-consuming [ （ 117.94 ± 44.30） s] and interference effects （ 82.42 ± 43.49） of TMT （P 〈 0.05 or P 〈 0. 01 ）. It were significantly different in intelligence quotient, similar comparison ,abstract inference items of R＇ SPM （P 〈 0.05 ）. The B item time-consuming and interference effects of TMT were correlated positively to age, course of disease in HLD group（P〈 0.05 or P〈0.01 ）. The scores of comparative inference of R＇ SPM were correlated negatively to age, course of disease in HLD group （P〈0.05）. Conclusion Cognitive flexibility dysfunction were appearing in hepatic type HLD patients. Damages of frontal - striatal loop functions caused to minimal hepatic encephalopathy were capital mechanisms of cognitive flexibility dysfunction in hepatic type HLD patients.